doi:10.1093/humrep/dem420
Human Reproduction Vol.23, No.3 pp. 548–553, 2008
Advance Access publication on January 12, 2008
Cervicovaginal agenesis: spontaneous gestation at term
after previous reimplantation of the uterine corpus
in a neovagina: Case Report
P. Acién1,2,3, M.I. Acién1,2, F. Quereda1,2 and T. Santoyo1,2
1
Service of Obstetrics and Gynecology, University Hospital of San Juan, Campus of San Juan, Alicante, Spain; 2Department/Division of
Gynecology, Miguel Hernández University, Campus of San Juan, Alicante, Spain
3
Correspondence address. E-mail: [email protected]
We present an exceptional case of a patient with complete cervical atresia and total vaginal aplasia. After McIndoe
vaginoplasty, cervicoitsmic resection with implantation and reimplantation of the uterine corpus in the neovagina,
and conservative surgery for endometriosis, the patient had normal menstruations and became pregnant spontaneously. A Caesarean section was performed at week 36. This is the third published case report of a successful spontaneous pregnancy and Caesarean section at term in a patient with complete cervicovaginal aplasia, and the first
published case study of a patient becoming pregnant after McIndoe vaginoplasty, cervicoistmic resection and
utero-neovaginal anastomosis. Gestation developed successfully without cerclage. We recommend conservative
surgery in patients with congenital cervical atresia. McIndoe vaginoplasty should be performed as soon as possible
in adolescence if there is associated vaginal aplasia. Nevertheless, fibrotic stenosis can occur, even after several
years and, therefore, additional operations and uterine reimplantation may be required.
Keywords: cervicovaginal agenesis; vaginal aplasia; Müllerian anomalies; McIndoe vaginoplasty; utero-neovaginal anastomosis
Introduction
Cervicovaginal agenesis is a complex Müllerian malformation
that generally presents with a normal functional uterus and
endometrium and apparently normal external genitals and
tubes. It is an uncommon and rare anomaly that causes
serious complications. In our opinion, this malformation
should be grouped with Müllerian anomalies that exhibit
failure or absence of the Müller tubercle (vagina) and of the
diverging portion of the Müller ducts (cervical atresia)
(Acién et al., 2004; Sánchez-Ferrer et al., 2006). Class Ia of
the ASRM Classification for uterine anomalies (American
Fertility Society, 1988) includes cases with vaginal agenesis
coupled with a functional uterus and endometrium; the uterus
may be normal or present fusion or resorption defects and the
cervix may be present, absent or hypoplastic (Grimbizis
et al., 2004; Sparac et al., 2004). Cases with cervical agenesis
are in Class Ib. Therefore, the true incidence of congenital cervical atresia is somewhat difficult to determine. Partial or complete cervical atresia has been described in the presence or
absence of vaginal atresia. A relatively common Müllerian
anomaly, such as congenital Müllerian agenesis corresponding
to Mayer – Rokitansky – Kuster – Hauser syndrome, may occur
with variable degrees of upper reproductive tract development
that range from either one or both functioning cavitated hemiuteri to a rudimentary uterus to the complete absence of
548
Müllerian tissue (Bryan et al., 1949; Jones and Wheeless,
1969; Griffin et al., 1976). The frequency of congenital
vaginal atresia is reportedly 1 in 4000 to 1 in 10 500 female
births (Griffin et al., 1976), with normal development of
the uterine corpus observed in ,10% of these patients.
However, the reported cases with congenital cervical atresia
are frequently associated with partial or minimal vaginal
aplasia of the upper part and cases with complete vaginal
aplasia usually present partial or minimal low cervical
atresia. Complete cervical and vaginal atresia is very rare. To
that end, very few reported cases have been identified. After
puberty, primary amenorrhoea may be the only symptom of
cervicovaginal atresia, but there is usually cyclic pelvic pain
that worsens with time. Physical examination generally
reveals normal external genitals, the absence of a vagina and,
in the rectal examination (and transrectal ultrasound), a
normal or thickened uterus, with or without pelvic tumours corresponding to hematometra, hematosalpinx or endometriosis.
There may be important post-surgery complications and, as
the reproductive capacity is low despite successful creation
of a neovagina, a hysterectomy is recommended as an elective
procedure, even in young patients (Niver et al., 1980; Rock
et al., 1984). Despite this recommendation, conservative
surgery is now becoming more frequently recommended in
patients with congenital cervical atresia and with total or
# The Author 2008. Published by Oxford University Press on behalf of the European Society of Human Reproduction and Embryology.
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Congenital cervicovaginal agenesis
Figure 1: (A) MR image
(B) Hysterosalpingography image. *Uterine cavity; X, misplaced
puncture and contrast injection
partial vaginal aplasia. In this communication, we present an
exceptional case of a patient with complete congenital cervical
atresia and vaginal aplasia who, after neovagina creation and
implantation and reimplantation of the uterine corpus in this
neovagina, had normal menstruations and became pregnant
spontaneously. A Caesarean section was successfully performed at week 36.
Case report
A fourteen-year-old girl who experienced primary amenorrhoea and cyclic pelvic pain was sent to our hospital in 1992
with a diagnosis of vaginal agenesis. The clinical examination
showed appropriate general feminization, normal external genitals and the absence of a vagina. The rectal exam revealed
thickening of the uterus and left adnex. The ultrasound evidenced the presence of uterine and left adnexal cysts. Urography indicated that both kidneys were normal. MRI (Fig. 1A)
revealed that the uterus and endometrial cavity were normal
with complete cervicovaginal agenesis. As dictated by the
above results, the following surgeries were planned:
(i) Diagnostic laparoscopy to confirm the presence of a
normal uterus and adnexes, and showing adhesions
and endometriomas on the left side. Through laparoscopic puncture of the uterus, a contrast agent was
introduced and an hysterosalpingography image was
obtained (Fig. 1B). Hematometra was not observed.
Figure 2: Diagram of the malformation and its correction
(A) RM and HSG images. (B) Congenital cervical atresia; lines and
areas of cervical resection. (a) resection level in 1992; (b) resection
level in 1993 and (c) resection level in 2002. (C) Uteroneovaginal
anastomosis
The uterine cervical portion and the right tube were
not filled, but the left tube was filled and indicated permeability and normal peritoneal spillage, although it
was distorted by the adhesions and endometriomas.
(ii) Creation of a neovagina, according to the McIndoe
technique, using a Dexon prosthesis and skin graft
taken from the right buttock.
(iii) Laparotomy, bladder separation, traction with Pozzi
tenacula of the atretic cervical tract, opening of the
neoformed vaginal fundus, exposing the upper side of
the prosthesis and resection of the cervical fibrous
tract without injuring the uterine vessels (see diagram
in Fig. 2). Cervical drilling was performed on the
upper side until the endometrial cavity was penetrated.
A vesical sound was introduced into the uterus and
vaginal extraction was performed via the light of the
Dexon prosthesis. Implantation of the uterine corpus
in the neovagina required suturing of the myometrium
and perimetrium to the edges of the buttonhole opened
on the neovaginal fundus. Adhesiolysis and conservative surgery for endometriosis were performed.
The post-operative period was normal. Antibiotic treatment,
endouterine sound and the prosthesis were maintained for nine
days. Following this period, the prosthesis and sound were
removed, the neovagina was made aseptic and the prosthesis
549
Acién et al.
was replaced. The patient was discharged from the Hospital
with the indication of placing and keeping the prosthesis in
the neovagina every night. The neovagina developed proper
squamous epithelium. However, the scar on the utero-vaginal
fundus soon became stenous and, after 2 – 3 menstruations,
amenorrhoea and cyclic dysmenorrhea reappeared. Therefore,
some months later (1993), the laparotomic surgery, a higher
cervical resection and reimplantation of the uterine corpus on
the neoformed vaginal fundus, now properly epithelized and
consolidated, were repeated. Afterwards, the patient had
cyclic menstrual bleeding and normal ultrasound check-ups
for 3 – 4 years; however, in 1998, after 2 years without checkups, she had strong post-menstrual abdominal pain and was
admitted to the Emergency Unit of another hospital where
she was diagnosed with breakage of endometriomas. A laparotomy and “partial resection of both ovaries with endometriomas” were performed. A posterior check-up in our Hospital
in November 1998 was normal, with a normal transvaginal
ultrasound, normal levels of CA-125 and normal cyclic menstrual bleeding.
In 2001, she presented with scarce menstrual bleeding, intermenstrual spotting and dysmenorrhea. In the transvaginal ultrasound, post-menstrual hematometra and some cysts suggestive
of adhesions on the left adnex were observed. As the menstruations were becoming scarcer and the dysmenorrhea stronger, a
new laparotomic surgery was performed. This surgery, which
was performed in January 2002, involved lysis of the adhesions, wide opening of the vaginal fundus, cone-shape resection higher in the uterus (Fig. 3) with progressive sections of
the low uterine portion up to the endometrium and uterine
Figure 3: Images of the last operation
Resection of the cervical portion (A) and reimplantation of the uterus
on the neoformed vaginal fundus (B)
550
cavity and reimplantation or utero-neovaginal anastomosis.
An endometrial biopsy was normal (secreting endometrium)
and a conservative surgery for endometriosis was performed.
After this last operation, normal cyclic menstrual bleeding
returned, dysmenorrhea disappeared and the yearly ultrasound
check-ups were normal. She had normal intercourse and
became pregnant spontaneously in May of 2006. She was constantly observed during the pregnancy. Cervical cerclage was
not done because of the absence of a cervix. The gestation
reached week 36 and, in January 2007, after a normal gestational ultrasound and documentation of a fetal weight of
3000 g, we performed a Caesarean section (Fig. 4). The
normal newborn was born with normal posterior evolution.
The transvaginal ultrasound check-up 2 months after the Caesarean section showed a normal uterus and endometrium and
absence of the uterine cervical portion.
Comments
As mentioned above, complete atresia of the vagina and cervical uterine portion with a functioning endometrial cavity is a
rare and complex anomaly. Sometimes, there is also uterine
or associated tubal malformation and, naturally, endometriosis,
depending on the time with respect to retrograde menstrual
bleeding and cryptomenorrhoea. In our case, we could not
confirm the permeability of the right tube, which may have segmentary atresia. We noticed and resected endometriomas and
adhesions on the left side each time that we performed a laparotomy. In addition, the report provided when she was laparotomized in a different hospital (in 1998) due to pain and possible
breakage of endometriomas states that “partial resection of
both ovaries with endometriomas” was performed. Given
these frequent alterations associated with the cervicovaginal
agenesis, the risk of ascending infections and the low reproductive capacity despite successful creation of a neovagina, hysterectomy is recommended as an elective procedure, even in
young patients. In cases of cervical agenesis, attempts to
make the cervix permeable usually fail, patients generally
have serious complications and normal menstruations and posterior pregnancy are rarely achieved (Niver et al., 1980; Rock
et al., 1984). However, in our case, despite the need of several
operations (reimplantation of uterine corpus in the neovagina
three times), menstrual bleeding remained normal for several
years, there have not been any ascending infections, and the
woman became spontaneously pregnant. We did not think
that gestation could be maintained for longer than 3 or 4
months due to the absence of the cervix and uterine closing
of a cervical cerclage; however, gestation continued normally
up to week 36. At this point, with a proper fetal weight, we performed a Caesarean section at the lower edge of the present
uterine corpus.
The creation of a neovagina and uterine opening or anastomosis into the neovagina should be done as soon as possible
(at 12 – 14 years) to avoid endometriosis. It may be preferable
to perform these procedures in two stages (Bugmann et al.,
2002). That is, the utero-vaginal anastomosis should be performed after the neovagina is epithelized and consolidated.
For vaginoplasty, we believe that the McIndoe technique is
Congenital cervicovaginal agenesis
Figure 4: Caesarean section
(A) The lower part of the pregnant uterus. (B) Fetus extraction. (C) Hysterorrhaphy
optimal, using an inert material prosthesis and skin graft. Other
techniques (such as Vecchietti’s, or using sigmoid bowel,
bladder mucous, peritoneum, etc.) are currently recommended,
but our experience in limited to the McIndoe technique. We
now use this technique with Interceed between the skin and
prosthesis and have good results in all cases. Nevertheless, if
a hysterectomy is required due to associated pathology, creation of the neovagina must be postponed until the age of
18 –20 years, at which time, surgery is technically easier, the
patient is more mature and the surgery can be followed by
sexual intercourse.
After serious complications in their cases, including death,
Geary and Weed (1973) realized that the patients with cervical
agenesis (and those with cervical atresia) required definitive
surgery (hysterectomy). Further, they suggested that creation
of an artificial duct would allow for, rather than inhibit, an
ascending infection and that the absence of cervical mucus
would make conception highly improbable. For these
reasons, heroic surgical efforts to preserve fertility would
not be justified in most patients, especially if cervical
and vaginal agenesis co-exist (Butram and Reiter, 1987).
However, as observed in our case report, cervicovaginal
atresia can be treated with surgery, with respect to menstrual
bleeding and posterior pregnancy. Sing and Devi (1983) communicated a case in which they had performed surgical reconstruction of the uterus and vagina. Despite the occurrence of
fibrotic stenosis in this case, a utero-vaginal fistula remained
and allowed for a successful posterior pregnancy. Also, successful pregnancies were reported by Welker et al. (1988)
after utero-vaginal anastomosis and by Hampton et al. (1990)
after vaginoplasty and cervical drilling; there was atresia of
the upper part of the vagina in the former case and vaginal
and partial agenesis of the cervical portion in the latter.
Fujimoto et al. (1997) revised the 58 cases of congenital cervical atresia reported in the literature, including their own 7
patients. Of these patients, 48% had isolated congenital cervical atresia with a normal vagina, whereas the remainder had
either complete or partial vaginal atresia. Normal menstrual
bleeding was achieved in 59% of the patients who underwent
utero-vaginal canalization procedures (23/39). Four of these
patients subsequently became pregnant and were delivered at
term. They conclude that surgical canalization in selected patients
with congenital cervical atresia can be successfully performed to
provide an opportunity for conservative management, resulting in
normal menstrual bleeding, resolution of cyclic pelvic pain, and
some potential for fertility. However, posterior fibrotic stenosis
usually occurs and further operations should be anticipated.
Deffarges et al. (2001) performed utero-vaginal or uterovestibular anastomosis in 18 patients with uterine cervix
atresia of whom 7 had associated high vaginal aplasia. A secondary stenosis of the anastomosis occurred in one case, and
two out of the seven cases with vaginal aplasia had successful
pregnancies. Likewise, Chakravarty et al. (2000) operated on
18 women with congenital cervicovaginal atresia using a new
technique of surgical canalization (vertical utero-cervical
incision by laparotomy, IUD as a cervical stent, anastomosis
to vault the vagina and vaginoplasty with a plastic mold and
amniotic membrane). Two of the cases had posterior pregnancies, but only one of them had complete cervicovaginal atresia.
More recently, laparoscopically-assisted utero-vestibular anastomosis was reported by Creighton et al. (2006) for 1 case
and by Fedele et al. (2007) for 12 cases with uterine cervix
atresia and vaginal aplasia. So far, these patients have not
become pregnant. We believe that utero-vaginal anastomosis
is an appropriate method in cases of cervical atresia or
atresia in the upper vagina, but, in case of vaginal aplasia, it
seems preferable to perform a vaginoplasty first.
Nine published cases of successful pregnancies in women
with cervicovaginal atresia are found in the literature; they
are summarized in Table I. There are only three cases (apart
from ours) with complete cervicovaginal atresia. Nargund
and Parsons (1996) reported one of these cases. Pregnancy
was achieved by IVF through the utero-vaginal fistula. Sing
and Devi’s case (1983) presented fibrotic stenosis, but a uterovaginal fistula remained, which allowed for a successful pregnancy. The case reported by Chakravarty et al. (2000) became
551
Acién et al.
Table I. Published cases of successful pregnancies in women with cervicovaginal atresia.
Reference
Cases with
cervicovaginal atresia
or agenesis
Surgery
Associated pelvic
lesions or genital
malformations
Associated pelvic
lesions in patients
who became pregant
Successful
pregnancies
Delivery
(Singh and
Devi, 1983)
1
–
Nonfused Müllerian
system
1, spontaneous
CS at term
(Welker et al.,
1988)
1, partial cervical
atresia, cervix
markedly dilated;
atresia of the upper
vagina
1, vaginal agenesis
and partial cervical
atresia; cervix
markedly dilated
1
Surgical reconstruction of the
uterus and vagina. Fibrotic
stenosis. Uterovaginal fistula
Utero-vaginal anastomosis
0
0
1, spontaneous
CS at 31
weeks
McIndoe vaginoplasty and
cervical drilling, cruciate
incisions
–
0
1, spontaneous.
Abdominal cerclage
at 12 weeks
CS at 38
weeks
McIndoe vaginoplasty and
cervical drilling failed, fistula
Ectopic ureter,
vesical atresia,
1, IVF
CS at 32
weeks
Vertical utero-cervical incision,
IUD Multiload as cervical
stent, fixation to vault of the
vagina, vaginoplasty with
plastic mold and amniotic
membrane
Utero-vaginal or vestibular
anastomosis
McIndoe vaginoplasty,
cervicoistmic resection and
reimplantation of the uterine
corpus in neovagina
?
Right tubo-ovarian
abcess,
adnexectomy
?
(2 spontaneous), “one
of whom had
complete
cervicovaginal
atresia”
Delivery of
viable
neonate.?
2, cases 8 and 9,
spontaneous
1, spontaneous
CS at 36–
38 weeks
CS at 36
weeks
(Hampton
et al., 1990)
(Nargund and
Parsons, 1996)
(Chakravarty
et al., 2000)
8
(Deffarges
et al., 2001)
Our case report
7 with high vaginal
aplasia
1, complete cervical
atresia and vaginal
aplasia
pregnant after vertical utero-cervical incision, anastomosis and
vaginoplasty with a plastic mold and amniotic membrane.
Therefore, our case is the third published case with a spontaneous successful pregnancy and Caesarean section at term
in a patient treated for complete cervicovaginal atresia. It is
the first case of a successful pregnancy after McIndoe vaginoplasty, cervicoisthmic resection and reimplantation of the
uterine corpus in neovagina (utero-neovaginal anastomosis).
She has required repeated operations, but has had menstrual
bleeding for 15 years and a spontaneous successful gestation
without cerclage or complications.
Therefore, we recommend conservative surgery in patients
with congenital cervical atresia, including the performance of
McIndoe vaginoplasty as soon as possible. This should be done
in adolescence if there is associated vaginal aplasia, with resection
of the atretic cervix and implantation and anastomosis of the
uterine corpus to the neovagina once the neovagina is properly
consolidated and epithelized. Despite these measures, stenosis
can occur in later years and, therefore, further operations and
uterine reimplantation should be anticipated.
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Submitted on September 6, 2007; resubmitted on November 30, 2007; accepted
on December 13, 2007
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